Free Online Decision Tools to Support Parents Making Decisions About Their Children's Chronic Health Condition: An Environmental Scan.

BACKGROUND: Medical decisions parents make on their children's behalf can be challenging. Free online decision support tools are created to help parents faced with these decisions. OBJECTIVE: We used an environmental scan to identify free, online tools that support parents in making decisions about their children's chronic health condition. We described the tools and assessed their potential to harm, content, development process, readability, and whether their use changed decision makers' knowledge and alignment of their preferences with their final decision. DATA SOURCES AND ELIGIBILITY: Decision aid repositories, Google searches, and key informants identified decision support tools. Eligible tools were freely available online and for parents of children with chronic health conditions. APPRAISAL METHODS: Two reviewers independently assessed the tools' quality based on the International Patient Decision Aid Standards (IPDAS). Tool readability was assessed using the Flesch Reading Ease test. RESULTS: From 21 free, online decision support tools, 14 (67%) provided sufficient detail for making a specific decision (IPDAS qualifying criteria). None sufficiently met IPDAS certification criteria necessary to reduce the possibility of patient harms when using the tool. Three (14%) were fairly easy or easy to read. Of those evaluated by developers (n = 6), 2 improved knowledge and 4 improved alignment of preferences with the available options. LIMITATIONS: Google searches and key informant sources are not replicable. CONCLUSIONS: Free, online decision support tools for parents of children with chronic health conditions are of variable quality, most are difficult to read, and there is limited evidence their use achieves intended outcomes. REGISTRATION NUMBER: Registered with Open Science Framework 20 July 2021(AEST) osf.io/b94yj.

Pulmonological issues.

Pediatric leukodystrophies are rare neurodegenerative diseases involving multiple systems. Each form has unique neurologic features but are characterized by encephalopathy with accompanying impairments evidenced in reflexes, muscle tone and movement control. Weakness of expiratory, inspiratory, and upper airway muscles may lead to impaired airway secretion clearance resulting in recurrent respiratory infections, dysphagia, sleep-disordered breathing, restrictive lung disease, and ultimately chronic respiratory insufficiency.

Barriers and Facilitators to the Implementation of Family-Centered Technology in Complex Care: Feasibility Study.

BACKGROUND: Care coordination is challenging but crucial for children with medical complexity (CMC). Technology-based solutions are increasingly prevalent but little is known about how to successfully deploy them in the care of CMC. OBJECTIVE: The aim of this study was to assess the feasibility and acceptability of GoalKeeper (GK), an internet-based system for eliciting and monitoring family-centered goals for CMC, and to identify barriers and facilitators to implementation. METHODS: We used the Consolidated Framework for Implementation Research (CFIR) to explore the barriers and facilitators to the implementation of GK as part of a clinical trial of GK in ambulatory clinics at a children's hospital (NCT03620071). The study was conducted in 3 phases: preimplementation, implementation (trial), and postimplementation. For the trial, we recruited providers at participating clinics and English-speaking parents of CMC<12 years of age with home internet access. All participants used GK during an initial clinic visit and for 3 months after. We conducted preimplementation focus groups and postimplementation semistructured exit interviews using the CFIR interview guide. Participant exit surveys assessed GK feasibility and acceptability on a 5-point Likert scale. For each interview, 3 independent coders used content analysis and serial coding reviews based on the CFIR qualitative analytic plan and assigned quantitative ratings to each CFIR construct (-2 strong barrier to +2 strong facilitator). RESULTS: Preimplementation focus groups included 2 parents (1 male participant and 1 female participant) and 3 providers (1 in complex care, 1 in clinical informatics, and 1 in neurology). From focus groups, we developed 3 implementation strategies: education (parents: 5-minute demo; providers: 30-minute tutorial and 5-minute video on use in a clinic visit; both: instructional manual), tech support (in-person, virtual), and automated email reminders for parents. For implementation (April 1, 2019, to December 21, 2020), we enrolled 11 providers (7 female participants, 5 in complex care) and 35 parents (mean age 38.3, SD 7.8 years; n=28, 80% female; n=17, 49% Caucasian; n=16, 46% Hispanic; and n=30, 86% at least some college). One parent-provider pair did not use GK in the clinic visit, and few used GK after the visit. In 18 parent and 9 provider exit interviews, the key facilitators were shared goal setting, GK's internet accessibility and email reminders (parents), and GK's ability to set long-term goals and use at the end of visits (providers). A key barrier was GK's lack of integration into the electronic health record or patient portal. Most parents (13/19) and providers (6/9) would recommend GK to their peers. CONCLUSIONS: Family-centered technologies like GK are feasible and acceptable for the care of CMC, but sustained use depends on integration into electronic health records. TRIAL REGISTRATION: ClinicalTrials.gov NCT03620071; https://clinicaltrials.gov/ct2/show/NCT03620071.

Intergenerational Decision Making: The Role of Family Relationships in Medical Decision Making.

A symposium held at the 42nd annual Society for Medical Decision Making conference on October 26, 2020, focused on intergenerational decision making. The symposium covered existing research and clinical experiences using formal presentations and moderated discussion and was attended by 43 people. Presentations focused on the roles of pediatric patients in decision making, caregiver decision making for a child with complex medical needs, caregiver involvement in advanced care planning, and the inclusion of spillover effects in economic evaluations. The moderated discussion, summarized in this article, highlighted existing resources and gaps in intergenerational decision making in four areas: decision aids, economic evaluation, participant perspectives, and measures. Intergenerational decision making is an understudied and poorly understood aspect of medical decision making that requires particular attention as our society ages and technological advances provide new innovations for life-sustaining measures across all stages of the lifespan.

Gastrostomy Tubes Placed in Children With Neurologic Impairment: Associated Morbidity and Mortality.

BACKGROUND: Gastrostomy tube (G-tube) placement for children with neurologic impairment with dysphagia has been suggested for pneumonia prevention. However, prior studies demonstrated an association between G-tube placement and increased risk of pneumonia. We evaluate the association between timing of G-tube placement and death or severe pneumonia in children with neurologic impairment. METHODS: We included all children enrolled in California Children's Services between July 1, 2009, and June 30, 2014, with neurologic impairment and 1 pneumonia hospitalization. Prior to analysis, children with new G-tubes and those without were 1:2 propensity score matched on sociodemographics, medical complexity, and severity of index hospitalization. We used a time-varying Cox proportional hazard model for subsequent death or composite outcome of death or severe pneumonia to compare those with new G-tubes vs those without, adjusting for covariates described above. RESULTS: A total of 2490 children met eligibility criteria, of whom 219 (9%) died and 789 (32%) had severe pneumonia. Compared to children without G-tubes, children with new G-tubes had decreased risk of death (hazard ratio [HR] 0.47, 95% confidence interval [CI] 0.39-0.55) but increased risk of the composite outcome (HR 1.21, CI 1.14-1.27). Sensitivity analyses using varied time criteria for definitions of G-tube and outcome found that more recent G-tube placement had greater associated risk reduction for death but increased risk of severe pneumonia. CONCLUSION: Recent G-tube placement is associated with reduced risk of death but increased risk of severe pneumonia. Decisions to place G-tubes for pulmonary indications in children with neurologic impairment should weigh the impact of severe pneumonia on quality of life.

Parent Perspectives in Shared Decision-Making for Children With Medical Complexity.

OBJECTIVE: Shared decision-making (SDM) may improve outcomes for children with medical complexity (CMC). CMC have lower rates of SDM than other children, but little is known about how to improve SDM for CMC. The objective of this study is to describe parent perspectives of SDM for CMC and identify opportunities to improve elements of SDM specific to this vulnerable population. METHODS: Interviews with parents of CMC explored SDM preferences and experiences. Eligible parents were ≥18 years old, English- or Spanish-speaking, with a CMC <12 years old. Interviews were recorded, transcribed, and analyzed by independent coders for shared themes using modified grounded theory. Codes were developed using an iterative process, beginning with open-coding of a subset of transcripts followed by discussion with all team members, and distillation into preliminary codes. Subsequent coding reviews were conducted until no new themes emerged and existing themes were fully explored. RESULTS: We conducted interviews with 32 parents (27 in English, mean parent age 34 years, standard deviation = 7; mean child age 4 years, standard deviation = 4; 50% with household income <$50,000, 47% with low health literacy) in inpatient and outpatient settings. Three categories of themes emerged: participant, knowledge, and context. Key opportunities to improve SDM included: providing a shared decision timeline, purposefully integrating patient preferences and values, and addressing uncertainty in decisions. CONCLUSION: Our results provide insight into parent experiences with SDM for CMC. We identified unique opportunities to improve SDM for CMC that will inform future research and interventions to improve SDM for CMC.

Health and Economic Outcomes of Posterior Spinal Fusion for Children With Neuromuscular Scoliosis.

OBJECTIVES: Neuromuscular scoliosis (NMS) can result in severe disability. Nonoperative management minimally slows scoliosis progression, but operative management with posterior spinal fusion (PSF) carries high risks of morbidity and mortality. In this study, we compare health and economic outcomes of PSF to nonoperative management for children with NMS to identify opportunities to improve care. METHODS: We performed a cost-effectiveness analysis. Our decision analytic model included patients aged 5 to 20 years with NMS and a Cobb angle ≥50°, with a base case of 15-year-old patients. We estimated costs, life expectancy, quality-adjusted life-years (QALYs), and incremental cost-effectiveness from published literature and conducted sensitivity analyses on all model inputs. RESULTS: We estimated that PSF resulted in modestly decreased discounted life expectancy (10.8 years) but longer quality-adjusted life expectancy (4.84 QALYs) than nonoperative management (11.2 years; 3.21 QALYs). PSF costs $75 400 per patient. Under base-case assumptions, PSF costs $50 100 per QALY gained. Our findings were sensitive to quality of life (QoL) and life expectancy, with PSF favored if it significantly increased QoL. CONCLUSIONS: In patients with NMS, whether PSF is cost-effective depends strongly on the degree to which QoL improved, with larger improvements when NMS is the primary cause of debility, but limited data on QoL and life expectancy preclude a definitive assessment. Improved patient-centered outcome assessments are essential to understanding the effectiveness of NMS treatment alternatives. Because the degree to which PSF influences QoL substantially impacts health outcomes and varies by patient, clinicians should consider shared decision-making during PSF-related consultations.

Trends in EMR for nonmalignant colorectal polyps in the United States.

BACKGROUND AND AIMS: Although most large nonpedunculated colorectal lesions can be safely and efficaciously removed using EMR, the use of colectomy for benign colorectal lesions appears to be increasing. The reason(s) is unclear. We aimed to determine the use and adverse events of EMR in the United States. METHODS: We used Optum's de-identified Clinformatics Data Mart Database (2003-2016), a database from a large national insurance provider, to identify all colonoscopies performed with either EMR or simple polypectomy on adult patients from January 1, 2011 to December 31, 2015. We measured time trends, regional variation, and adverse event rates. We assessed risk factors for adverse events using multivariate logistic regression. RESULTS: The rate of EMR use in the US increased from 1.62% of all colonoscopies in 2011 to 2.48% of colonoscopies in 2015 (P < .001). There were, however, significant regional differences in the use of EMRs, from 2.4% of colonoscopies in the western United States to 2.0% of colonoscopies in the southern United States. Between 2011 and 2015, we found stable rates of perforation, GI bleeding (GIB), infections, and cardiac adverse events and decreasing rates of admissions after EMR. In our multivariate model, EMR was an independent risk factor for adverse events, albeit the rates of adverse events were low (1.35% GIB, .22% perforation). CONCLUSIONS: Use of EMR is rising in the United States, although there is significant regional variation. The rates of adverse events after EMR and polypectomies were low and stable, confirming the continued safety of EMR procedures. A better understanding of the regional barriers and facilitators may improve the use of EMR as the standard management for benign colorectal lesions throughout the United States.

Pneumonia Prevention Strategies for Children With Neurologic Impairment.

BACKGROUND: Children with neurologic impairment (NI) face high risk of recurrent severe pneumonia, with prevention strategies of unknown effectiveness. We evaluated the comparative effectiveness of secondary prevention strategies for severe pneumonia in children with NI. METHODS: We included children enrolled in California Children's Services between July 1, 2009, and June 30, 2014, with NI and 1 pneumonia hospitalization. We examined associations between subsequent pneumonia hospitalization and expert-recommended prevention strategies: dental care, oral secretion management, gastric acid suppression, gastrostomy tube placement, chest physiotherapy, outpatient antibiotics before index hospitalization, and clinic visit before or after index hospitalization. We used a 1:2 propensity score matched model to adjust for covariates, including sociodemographics, medical complexity, and severity of index hospitalization. RESULTS: Among 3632 children with NI and index pneumonia hospitalization, 1362 (37.5%) had subsequent pneumonia hospitalization. Only dental care was associated with decreased risk of subsequent pneumonia hospitalization (adjusted odds ratio [aOR]: 0.64; 95% confidence interval [CI]: 0.49-0.85). Exposures associated with increased risk included gastrostomy tube placement (aOR: 2.15; 95% CI: 1.63-2.85), chest physiotherapy (aOR: 2.03; 95% CI: 1.29-3.20), outpatient antibiotics before hospitalization (aOR: 1.42; 95% CI: 1.06-1.92), clinic visit before (aOR: 1.30; 95% CI: 1.11-1.52), and after index hospitalization (aOR: 1.72; 95% CI: 1.35-2.20). CONCLUSIONS: Dental care was associated with decreased recurrence of severe pneumonia. Several strategies, including gastrostomy tube placement, were associated with increased recurrence, possibly due to unresolved confounding by indication. Our results support a clinical trial of dental care to prevent severe pneumonia in children with NI.

Potential Impact of Initial Clinical Data on Adjustment of Pediatric Readmission Rates.

OBJECTIVE: Comparison of readmission rates requires adjustment for case-mix (ie, differences in patient populations), but previously only claims data were available for this purpose. We examined whether incorporation of relatively readily available clinical data improves prediction of pediatric readmissions and thus might enhance case-mix adjustment. METHODS: We examined 30-day readmissions using claims and electronic health record data for patients ≤18 years and 29 days of age who were admitted to 3 children's hospitals from February 2011 to February 2014. Using the Pediatric All-Condition Readmission Measure and starting with a model including age, gender, chronic conditions, and primary diagnosis, we examined whether the addition of initial vital sign and laboratory data improved model performance. We employed machine learning to evaluate the same variables, using the L2-regularized logistic regression with cost-sensitive learning and convolutional neural network. RESULTS: Controlling for the core model variables, low red blood cell count and mean corpuscular hemoglobin concentration and high red cell distribution width were associated with greater readmission risk, as were certain interactions between laboratory and chronic condition variables. However, the C-statistic (0.722 vs 0.713) and McFadden's pseudo R2 (0.085 vs 0.076) for this and the core model were similar, suggesting minimal improvement in performance. In machine learning analyses, the F-measure (harmonic mean of sensitivity and positive predictive value) was similar for the best-performing model (containing all variables) and core model (0.250 vs 0.243). CONCLUSIONS: Readily available clinical variables do not meaningfully improve the prediction of pediatric readmissions and would be unlikely to enhance case-mix adjustment unless their distributions varied widely across hospitals.

Well-Appearing Newborn With a Vesiculobullous Rash at Birth.

A term, appropriate-for-gestational-age, male infant born via normal spontaneous vaginal delivery presented at birth with a full-body erythematous, vesiculobullous rash. He was well-appearing with normal vital signs and hypoglycemia that quickly resolved. His father had a history of herpes labialis. His mother had an episode of herpes zoster during pregnancy and a prolonged rupture of membranes that was adequately treated. The patient underwent a sepsis workup, including 2 attempted but unsuccessful lumbar punctures, and was started on broad-spectrum antibiotics and acyclovir, given concerns about bacterial or viral infection. The rash evolved over the course of several days. Subsequent workup, with particular attention to his history and presentation, led to his diagnosis.

Shared Decision Making among Children with Medical Complexity: Results from a Population-Based Survey.

OBJECTIVE: To compare the rates of shared decision making (SDM) reported by parents of children with medical complexity (CMC) with the rates of SDM reported by parents of noncomplex children with special health care needs (CSHCN). STUDY DESIGN: We examined the 2009-2010 National Survey of Children with Special Health Care Needs, a representative survey of 40 242 parents of CSHCN. CMC was defined as needing or using more medical care than usual, seeing 2 or more subspecialists, and positive response on at least 3 other items on the CSHCN screener. We identified 3 subgroups each of CMC and noncomplex CSHCN by sentinel diagnoses: asthma, seizures, and other diagnoses. SDM was defined as a binary composite variable, derived from 4 discrete items. We constructed 4 stepwise multivariable models to assess the relative odds of SDM, adjusted for sociodemographic characteristics (age, income, language, race, ethnicity, and marital status), behavioral comorbidity, family-centered care, and patient-centered medical home. RESULTS: The study population included 39 876 respondents. Compared with noncomplex CSHCN, CMC had a lower likelihood of SDM (aOR, 0.76; 95% CI, 0.64-0.91), which persisted in diagnostic subgroups: CMC with asthma (aOR, 0.67; 95% CI, 0.49-0.92) and CMC with other diagnoses (aOR, 0.74; 95% CI, 0.58-0.94), but not CMC with seizures (aOR, 0.95; 95% CI, 0.59-1.51). CONCLUSIONS: SDM is less common for CSHCN with complex needs than those without complex needs. Health system interventions targeting future-oriented care planning may improve SDM for CMC.

Does Routine Imaging of Patients for Progression or Relapse Improve Survival in Rhabdomyosarcoma?

BACKGROUND: Patients with rhabdomyosarcoma (RMS) who complete therapy typically undergo 4 years of surveillance imaging despite lack of evidence that this improves outcomes. We compared overall survival (OS) between patients in whom progression or relapse was detected by routine clinical evaluation or by imaging. PROCEDURE: Children with progressive or relapsed RMS treated at Texas Children's Hospital between 1992 and 2012 were identified and their records were reviewed. Survival time after progression or relapse was compared between two groups: (1) patients in whom progression or relapse was suspected on the basis of clinical history, symptoms, laboratory evaluation, or physical exam; and (2) patients whose progression or relapse was initially detected by imaging. RESULTS: Of the 43 children with progressive or relapsed RMS, 26 (60%) had metastatic disease at diagnosis and 19 (44%) had alveolar histology. With a median follow up time of 5 years in six survivors, there was no difference in OS between patients in whom progression or relapse was diagnosed based on imaging (n = 15) or by clinical evaluation (n = 28) (3-year OS 20% vs. 11%, respectively, P = 0.38). Disease extent, primary site, and risk group at diagnosis were associated with survival after progression or relapse. CONCLUSIONS: Routine surveillance imaging practice should be critically reviewed for children with RMS. Although our findings must be validated by larger studies, they do have substantive implications. Reduced imaging tailored to the risk and pattern of recurrence, associated risks and cost could improve patient quality of life and decrease health-care expenditure without compromising outcome.